The UMD-FBN1 mutations database
Record ID: 600

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.1345G>Ap.Val449IleHeterozygousPolymorphism

wt codonwt aamutant codonmutant aamutational eventmutation type
GTTValATTIleG->ATs

StructureKey Residue (HCD)Pyrimidin doubletCpG
EGF-like #04 N-Term MMPs siteNoYes

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): Mae II

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.831.00 (non pathogenous)35 (Polymorphism)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
GER03HAN F0013 I03ProbandMaleNA15 years oldGERMANY

Phenotypic groupDisease
NAIncomplete MFS

Clinical data


SymptomSeverity
C-Aortic insufficiencysevere
C-Asc. aortic dilatation
no clinical data
O-No implication
S-Increased body length

Reference


Reference IDPubMed IDReference
10712402346
Rommel K, Karck M, Haverich A, Schmidtke J, Arslan-Kirchner M. "Mutation screening of the fibrillin-1 (FBN1) gene in 76 unrelated patients with Marfan syndrome or Marfanoid features leads to the identification of 11 novel and three previously reported mutations". Hum Mutat. 2002 Nov;20(5):406-7.